胰腺神经内分泌微瘤(2022年世界卫生组织分类)并非总是低级别肿瘤:一例增殖率显著升高的病例
Pancreatic Neuroendocrine Microtumors (WHO 2022) Are Not Always Low-Grade Neoplasms: A Case with a Highly Increased Proliferation Rate.
作者信息
Chouchane Aziz, Kirchner Philipp, Marinoni Ilaria, Sticová Eva, Jirásek Tomáš, Perren Aurel
机构信息
Institute For Tissue Medicine and Pathology, University of Bern, Bern, Switzerland.
Clinical and Transplant Pathology Centre, Institute of Clinical and Experimental Medicine, Prague, Czech Republic.
出版信息
Endocr Pathol. 2024 Jun;35(2):147-153. doi: 10.1007/s12022-024-09802-7. Epub 2024 Feb 26.
Traditionally considered non-functional low proliferative benign neuroendocrine proliferations measuring less than 5 mm, pancreatic (neuro)endocrine microadenomas are now classified as pancreatic neuroendocrine microtumors in the 2022 WHO classification of endocrine and neuroendocrine tumors. This case report discussed the features of an incidentally identified 4.7-mm glucagon-expressing pancreatic neuroendocrine microtumor with MEN1 mutation only, chromosomally stable and an epigenetic alpha-like phenotype. The tumor was associated with an unexplained increased proliferation rate in Ki-67 of 15%. There was no associated DAXX/ATRX deficiency. The presented case challenges the conventional thought of a low proliferative disease of the so-called "pancreatic neuroendocrine microadenomas" and provides additional support to the 2022 WHO classification that also requires grading of these neoplasms. Despite exhibiting molecular features of less aggressive behavior, the case also underscores the biological complexity of pancreatic neuroendocrine microtumors. By recognizing the heterogenous spectrum of neuroendocrine neoplasms, the current case also contributes to ongoing discussions on how to optimize the clinical management of such tumors.
胰腺(神经)内分泌微腺瘤传统上被认为是无功能的低增殖性良性神经内分泌增生,直径小于5毫米,在2022年世界卫生组织内分泌和神经内分泌肿瘤分类中,现在被归类为胰腺神经内分泌微肿瘤。本病例报告讨论了一例偶然发现的4.7毫米表达胰高血糖素的胰腺神经内分泌微肿瘤的特征,该肿瘤仅存在MEN1突变,染色体稳定,具有表观遗传α样表型。肿瘤伴有Ki-67增殖率不明原因地增加至15%。不存在相关的DAXX/ATRX缺陷。该病例挑战了所谓“胰腺神经内分泌微腺瘤”低增殖性疾病的传统观念,并为2022年世界卫生组织分类提供了额外支持,该分类也要求对这些肿瘤进行分级。尽管该病例表现出侵袭性较低行为的分子特征,但也强调了胰腺神经内分泌微肿瘤的生物学复杂性。通过认识到神经内分泌肿瘤的异质性谱,本病例也有助于正在进行的关于如何优化此类肿瘤临床管理的讨论。